Coping with Neuromuscular disease and the Implications for Interventions

The lecture is based on results from three theses and about 25 scientifically articles about the plight of the patient with muscular dystrophy (MD), and the patients’ ways of coping with illness-related problems from a psychosocial perspective. MD is a group of inherited disorders characterised by muscular weakness caused by muscle wasting. Three subgroups are distinguished in our research: one group with different types of proximal MD and the other two groups characterized chiefly by distal weakness. The research was to elucidate how persons with muscular dystrophy experience their everyday life over a ten years period. The research includes four data collections with qualitative and quantitative methods. The data was selected in three different county councils in Sweden.

The persons with MD described the change from being independent to being an individual in need of assistive devices and/or personal support to manage activities of every day life. Besides muscular weakness the persons have the strain it means to have a hereditary disease. The deterioration in a decennium was mainly with regard to ambulation. The number of persons walking without assistive devices and working has almost been halved. They experienced periods of sorrow due to losses of independence, control, status and social roles and the loss of one’s identity as a healthy person. The sorrow connecting with repeated losses are in the literature designate as chronic or episodic sorrow. Also, in the lecture the patients’ illness trajectory were described by a model of Sullivan (1994) into four dimensions in order to increase the understanding of what it means to live with muscular dystrophy.

The aim of interventions is to support the patient’s appropriate ways of coping as Problem-focused coping/Searching for a solution, Fighting spirit/Struggling, Re-appraising of life values, Maintaining hope, Social comparison, Laughing and joking and Caring about self.

6.

Ahlström, Gerd

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. HHJ. Quality improvements, innovations and leadership in health care and social work.

Aims and objectives. The aims of the present study were to describe losses narrated by persons afflicted with severe chronic physical illness and to identify the concomitant occurrence of chronic sorrow. Background. Reactions connected with repeated losses are referred to in the literature as chronic sorrow, which has recently been described in conjunction with chronic illness. Design. A qualitative study with an abductive approach of analysis, including both inductive and deductive interpretations. Method. The study is based on 30 persons of working age with average disease duration of 18 years. The average age was 51 years. All of the persons had personal assistance for at least three months because of considerable need for help in daily life due to physical disability. Each person was interviewed twice. There was also an independent assessment of the deductive results concerning chronic sorrow. Results. The inductive findings show that all persons had experienced repeated physical, emotional and social losses. Most common were 'Loss of bodily function', 'Loss of relationship', 'Loss of autonomous life' and 'Loss of the life imagined'. 'Loss of identity' included the loss of human worth, dignity and a changed self-image. In addition, the deductive findings suggest that chronic sorrow exists in the study population. Sixteen of 30 participating subjects were assessed by both assessors to be in a state of chronic sorrow and there was an especially high agreement with respect to one criterion of chronic sorrow 'Loss experience, ongoing or single event' (28 of 30 subjects). Conclusions. This study shows that persons with severe chronic illness often experience recurring losses. These experiences are consistent with the phenomenon of chronic sorrow. Relevance to clinical practice. Knowledge of the existence of chronic sorrow in persons with chronic illness will enable nurses to support these persons in a more sensitive and appropriate way.

We all need to start where we are and also wanting to go further all the time. This is the essence in quality improvement. The leader and the teachers must own this attitude themselves in order to have the ability to be trustworthy for the students. There is evidence in the literature about the impact of education on the professional attitude and role. This means that it is important to work with better professional development for better outcomes in the faculty.

One starting point for us in the planning of our different programs are that improvement knowledge will be a streak through the whole education in order to establish a professional attitude of daily inspiration to produce the best practise. We have applied all curricula to Boologna declaration and in this system progression in learning is a key point. We have four levels in the basic education (3-years education which lead to Bachelor degree) where we start to introduce 1) Concepts and models in health improvement. Then let our students make a 2) Personal improvement in their everyday life. Later in the education we teach about 3) Evidence practise and systematic literature reviews. Finally the students are 4) Making health improvements in collaboration with the staff during one clinical education.

All educational programmes on basic level since 2007 include aim descriptions about quality improvement. The aim of the learning is to have the competence to initiate and participate in work about health care improvements.

Finally, besides the mentions aspects in improvement education there are some other factors that needs for better system performance.

Since one of the aims of this meeting is to create a strategy for the participants to connect and share experiences post-conference I will start by giving a very brief account of Jönköping University and the School of Health Sciences, this in order to give you a sense of the school as a possible partner for collaboration. Then I will describe how teachers work to increase knowledge with regard to health care improvement within the nursing program. Thereafter I will talk about a research project and relate this to what we can learn about interprofessional education. The basic values regarding health care improvement described by Paul Batalden and his colleagues are central to our present development. We share the theoretical assumptions concerning the Clinical Microsystem and the view of redesigning health care education in order to produce the best care for the patient and his/her family. So within our organization both teachers and students — like nurses — have two jobs, one of which is to bring about improvement. So we have started the process where the educators need to change their teaching for the sake of better patient outcomes, better system performance, and better professional development. This means communicating not only the existing evidence-based knowledge but also improvement knowledge which involves a substantial shift in our idea of the work of health care — a challenging task that can benefit from the use of a wide variety of tools and methods. A key concern for us in the planning of our different programs is that the improvement of knowledge shall permeate the education and contribute to establishing a professional attitude marked by daily inspiration to produce the best care. The basic education involves four stages. First there is an introduction to concepts and models in health care improvement. This content is in the first semester. Second the students’ are making a personal improvement in their everyday life. The students apply PDSA-cycle for improvement of an issue, commonly their time-table for studies, time spending for eating breakfast in the morning or eating habits in general. The third stage involves evidence-based nursing and systematic literature reviews and in the fourth stage, the students make health care improvements in collaboration with the staff during a clinical training period. The nurses at the clinic formulate the area of improvement. Then the students work systematically in pairs to collect data and the results are reported to the nurses. The latter stage is a new content in nursing education but we know from occupational therapy that the staff appreciated these works and the results were in most cases used by the staff in their job.

The overall aim of the research project “Bridging the gaps” is to generate evidence about the process and outcome of clinical work within the area of quality improvement. The project contributes to an advanced environment for learning and innovation, research and development in the field of health-care management and improvement. There are 14 doctoral students at the research school, who takes part in the research project. Three of them are nurses and doctoral students at the School of Health Sciences. All PhD students have to take two compulsory courses, one is named “Bridging the gaps — a multidisciplinary research field” and the other is named “Interactive Research Methodology”. The Microsystem as a theoretical model is included in the mention research course “Bridging the gaps” and in each doctoral student’s research proposal. The PhD students shall work together with health-care staff by way of an interactive research method. This means that there is an integration of research and practice. The PhD students and the health-care staff are working together throughout the project. The staff is involved at every stage, from designing the improvement work, formulating the research questions to reporting the results. Research with this methodology generates knowledge of great relevance for clinical practice.

12.

Ahlström, Gerd

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. HHJ. Quality improvements, innovations and leadership in health care and social work.

Patients with neurological diseases increasingly are being cared for at home. The purpose of this study was to describe stressful everyday experiences in connection with neurological disorders, to learn how people cope with such experiences, and gain knowledge about coping resources. Twenty-seven persons receiving personal assistance were interviewed twice at home. The 54 interviews were subjected to inductive content analysis. The persons encountered many problems in everyday life and usually handled them by means of acceptance, avoidance, practical problem solving, and reappraisal. Dependence on personal assistance gave rise to a sense of helplessness, but it was also the major coping resource. Nurses can improve home care by giving instruction and guidance to persons working as personal assistants.

16.

Ahlström, Gerd

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. HHJ. Quality improvements, innovations and leadership in health care and social work.

The lecture will present an overview of the development and results from the Swedish version of the WCQ. The process of translation of the original questionnaire into Swedish is described as well as the results of the first Swedish study about coping with illness-related problems in persons with chronic diseases compared with a group healthy subjects. The first findings indicated the importance of further testing of the psychometric properties of the WCQ. Further psychometric evaluation of the WCQ included 510 subjects (patients, their next of kin and students). The result indicated modest support for the 8-factor model and deviation from equality of factor structures among the sub-samples. The 8-factor model was more adequate in describing clinical than non-clinical samples. A modification of the WCQ with a version of 45 items was made based on the results, which is now current in several studies.

18.

Ahlström, Gerd

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. HHJ. Quality improvements, innovations and leadership in health care and social work.

In the county of Orebro, Sweden, 32 individuals with myotonic disorders and 25 with other types of muscular dystrophy were examined. Disability was assessed with functional tests and standardized observations of muscle function (mainly based on those proposed by Dr. Brooke), a new self-administered questionnaire regarding the Activities of Daily Living (ADL) and the ADL staircase (based on Katz ADL index). The results of the different tests of disability were highly correlated. The Sickness Impact Profile and the Kaasa test were used for assessing the quality of life, and no significant differences were found between the groups of muscular dystrophy. In an explanatory factor analysis three main factors of disability were found. The factors "walk and move" and "finger function" were fair to good associated with the quality of life. This study offers an approach for research on the consequences of muscular dystrophy using established as well as new methods.

28.

Ahlström, Gerd

et al.

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. Jönköping University, School of Health Science, HHJ. Quality improvements, innovations and leadership in health care and social work.

All individuals in a Swedish county afflicted with any type of hereditary muscular dystrophy (MD) were identified and 57 (85 percent) of eligible individuals in the age range 16 to 64 were included in the study. Respiratory disturbances were estimated by means of spirometry and analysis of arterial blood gases, and 58 percent yielded abnormal results on at least one of these examinations. Elevated PCO2 was found more commonly than reduced forced vital capacity (FVC) and there was a moderate association between these parameters. Respiratory symptoms, most commonly breathlessness, were encountered in 79 percent. Pathologic ECG recordings were found in 21 individuals (37 percent). Conduction disturbances and affection of the myocard were most frequent in myotonic dystrophy. Quality of life was assessed by means of the Sickness Impact Profile instrument and the Kaasa test. The results showed that quality of life was significantly related to FVC and to the symptom of abnormal fatigue. Respiratory and cardiac parameters showed a greater number of significant correlations with measures of functional ability than with subjective well-being.

32.

Ahlström, Gerd

et al.

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. Jönköping University, School of Health Science, HHJ. Quality improvements, innovations and leadership in health care and social work.

The epidemiology of neuromuscular diseases was studied in the county of Orebro, Sweden (study population 270,000). Several different sources of data were utilized, compared and validated. On the prevalence of day (January 1, 1988) 474 patients were identified. The rate per 100,000 population was 92 for the postpolio sequelae (PPS) and 84 for the other neuromuscular diseases (motor neuron disease 9, hereditary neuropathies 9, myoneural disorders 16, myotonic disorders 19, muscular dystrophies 20 and myositis 11). Of the patients with the PPS, 80% reported late-onset symptoms. On the basis of an expanded survey including all medical records in one health care district, the prevalence of the PPS was estimated to be 186/100,000 population.

33.

Ahlström, Gerd

et al.

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. Jönköping University, School of Health Science, HHJ. Quality improvements, innovations and leadership in health care and social work.

PURPOSE: The purpose of the study is to investigate disability and quality of life in individuals with the characteristic symptoms of postpolio syndrome. METHOD: Disability is assessed by means of the self-report activities of daily living instrument, and quality of life by means of Kaasa's questionnaire and the quality of life profile. RESULTS: The 39 subjects have on average lived with polio sequelae for 52 years. Their main difficulties are with moving, lifting and carrying. This means restricted mobility, sedentary activities and a need to prioritize. Half of them feel that polio has lessened their possibilities in life, and a quarter have still not accepted the limitations polio has involved. Nevertheless the majority report a high level of psychosocial well-being, and almost a quarter say that living with polio has meant personal development and strength. We found a significant correlation between on the one hand disability with regard to ambulation, arm strength and finger strength on the self-report ADL, and on the other hand the number of negative problems on the quality of life profile (0.33-0.45). CONCLUSION: The latter instrument needs further testing before its validity can be determined with certainty.

35.

Ahlström, Gerd

et al.

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. Jönköping University, School of Health Science, HHJ. Quality improvements, innovations and leadership in health care and social work.

OBJECTIVE: To assess if activities of daily living (ADL), coping and quality of life could be improved in adults with muscular dystrophy through a comprehensive rehabilitation programme. DESIGN: Quasi-experimental, controlled clinical study comparing patients with similar age and disease aspects. SETTING: Two different counties in Sweden, being either study or control setting. SUBJECTS: The study group comprised 37 adults (21 women, 16 men; mean age 50 years), while the control group comprised 39 people (25 women, 14 men; mean age 46 years). INTERVENTIONS: Four rehabilitation sessions tailored to different medical, physical and psychosocial needs of the patients, comprising a total of 10 days over a period of 18 months. MAIN MEASURES: ADL, the Mental Adjustment to Cancer Scale measuring coping strategies, the Sickness Impact Profile measuring health-related quality of life, the Hospital Anxiety and Depression Scale, and the Psychosocial Well-being Questionnaire. RESULTS: No significant differences were found between groups with regard to the outcome measures. There was increased dependence on others in ADL after 18 months in both groups, but it was more pronounced in the control group. Furthermore, a clear trend was observed in the data with regard to coping patterns, the control group using more coping strategies such as 'Helplessness/hopelessness' (P= 0.057), 'Anxious preoccupation' (P = 0.085) and 'Fatalistic' (P= 0.073) when being compared to the study group. CONCLUSIONS: No apparent effects on ADL were found from the rehabilitation programme, although there was a tendency of reduction of maladaptive coping patterns in the study group. This initial study may provide the rationale and basis for a randomized controlled trial.

37.

Ahlström, Gerd

et al.

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. Jönköping University, School of Health Science, HHJ. Quality improvements, innovations and leadership in health care and social work.

Illness-related problems and coping were examined in 60 individuals with muscular dystrophy (MD) identified in a population survey of the county of Orebro, Sweden. In addition, the extent to which coping is related to quality of life (QoL) was investigated as was the impact of impairment and disability on the relation between coping and QoL. Emotion/appraisal-focused coping was utilized by respondents more than twice as often as problem-focused coping. High QoL was significantly correlated to "Stoic acceptance" and "Tried alternative treatment." Low QoL was associated with "Helpless/hopeless," "Anxious preoccupation," "Minimization," "Social comparison," "Establishment of control over everyday life," "Performs the task with the aid of an appliance" and "Accepts help or leaves it to others." When measures of impairment and disability were included in the analysis, the impact of these measures explained the association between coping and physical QoL by 16% to 43%.

There is no published research on coping with muscular dystrophy among adults. In the present study, two questionnaires, the Reaction to the Diagnosis of Cancer Questionnaire (RDCQ) and the Mental Adjustment to Cancer scale (MAC), were modified in order to measure coping with muscular dystrophy (MD). A total of 60 people (16-64 years) with diagnosed MD answered the questionnaires in two interviews including semi-structured questions. The replies to these questions were analysed by two independent judges on the basis of the RDCQ and MAC categories for coping. The purpose was twofold: to investigate if cancer-coping categories could be used for the classification of interview answers concerning coping with MD, and to gain knowledge about specific coping with MD. Analysis indicated that 82% of replies to semi-structured questions concerned with emotion/appraisal-focused coping with MD can be described by means of RDCQ and MAC categories. Eight new categories were developed to classify the remaining 18%: Anticipation, Creation of new life values, Minimization, Establishment of control over everyday life, Secretiveness, Fear, Social comparison and Coping with heredity. In addition, 997 replies were classified to represent problem-focused coping. The judges reached good agreement with respect to the proportions of replies in the respective coping categories. However, kappa (kappa) values were within the range of fair to good agreement.

39.

Ahlström, Gerd

et al.

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. HHJ. Quality improvements, innovations and leadership in health care and social work.

Abstract In Sweden adults with major and sustainable disabilities can be granted publicly financed support in the form of personal assistance after assessment and decision-making in accordance with government rules. The purpose of the present study was to explore more deeply the encounters in close care relations between personal assistants and disabled persons of working age, as well as the prerequisites for and obstacles to the success of such encounters, this from the perspective of the personal assistants. Thirty-two personal assistants (22- to 55-year old) who worked for 32 persons with serious neurological diseases living at home were interviewed. The transcribed unstructured interviews were qualitatively analysed using latent content analysis. The analyses resulted in five main themes: Perceptive awareness, Entering into the other's role, Mutuality, Handling the relationship and Personal difficulties facing the assistant. These themes illustrated that a prerequisite for the encounter's being meaningful is that the assistant should be able to observe and understand the unique needs of the disabled person. The assistant must furthermore be able to put herself/himself into the other person's position. It is also important that the personal chemistry between the assistant and the disabled person should be good. Being able to share feelings and interests with the functionally impaired person provides the assistant with positive emotional confirmation of a good relationship. A distressing dilemma the assistant faces is that of distinguishing between the working relationship and the personal friendship. In this borderline area are found experiences, feelings and events that the assistant may view as negative or even unacceptable. This study contributes to the understanding of the complexity underlying the daily community care of disabled persons with an extensive need for care and assistance.

One of the most widely used self-assessment inventories of coping is the Ways of Coping Questionnaire. The aims of the study were to describe coping with illness-related problems in persons with muscular diseases and to investigate the influence of demographic variables. Also, the reliability of the Swedish version of the Ways of Coping Questionnaire was to be evaluated. Forty-four persons with muscular dystrophy and 32 persons with postpolio syndrome, along with a comparison group of 214 students, completed the questionnaire. The findings of this study show that there are some different coping patterns in students as compared with persons with chronic disease. The coping strategy Distancing was used more and the strategy Accepting Responsibility used less frequently in the chronic disease groups and may be an expression of the inevitable physical limitations caused by the progressiveness of muscular dystrophy and postpolio syndrome. Furthermore, persons with muscular dystrophy also used less problem-focused coping in the form of Confrontive Coping and Planful Problem Solving when compared with the postpolio and student groups. In addition, women in the three groups employed the strategy of Seeking Social Support more often than did men. Regarding the psychometric properties of the Swedish version of the Ways of Coping Questionnaire, internal consistency was acceptable for the total score, but lower and not acceptable for several of the subscales. In the student group, but not in the chronic disease groups, there were low correlations between subscales, which indicate discriminative ability. More research is necessary to further investigate the reliability and validity of the Swedish version of the Ways of Coping Questionnaire before this instrument can be used routinely to assess coping patterns in patients with chronic disease.

Our aim in this study was to describe how patients perceive having recovered from eating disorders. A qualitative method with a phenomenographic approach was used to identify various ways of experiencing recovery. Four categories emerged, describing how the subjects now relate in a relaxed and accepting manner to food, the body, themselves as individuals, and their social environment. Some perceived recovery as coping with emotions, while others experienced themselves as healthier than people in general regarding food and weight. Different aspects were emphasized as important for recovery. As long as patients perceive themselves as recovered, it is not necessary that they fulfill all conceivable criteria for recovery.

48. Boo Murander, Margaretha

et al.

Ahlström, Gerd

Jönköping University, School of Health Science, HHJ, Dep. of Nursing Science. Jönköping University, School of Health Science, HHJ. Quality improvements, innovations and leadership in health care and social work.

PURPOSE: The aim of the study was to elucidate experiences of living with muscular dystrophy in terms of consequences for activity over 10 years. METHODS: The study population was identified in a prevalence study in a county of Sweden. Forty-six persons of this cohort with MD were interviewed. A qualitative research approach was chosen. The World Health Organization's International Classification of Functioning, Disability and Health (ICF) was used for categorization. RESULTS: Nearly all the subjects experienced a deterioration of physical capacity. Most obvious were the restrictions on mobility and increased fatigue and feebleness. The persons described psychosocial consequences of the muscular dystrophy as well as stigma when the disability had become more obvious. In spite of reported distress several persons experienced better psychological adaptation over time. The image of the future was often dark but several focus on today and avoid thoughts about the future. ICF showed some limitation with regard to temporal aspects, emotions and the subjective perspective. CONCLUSIONS: The knowledge of the trajectory with MD is important in order to offer the best possible treatment and support. Repeated assessment by ICF can serve as a valuable source of such knowledge, and a development of the classification would increase its usefulness in future analysis of functioning and disability.